Harold Ramis' death puts vasculitis in the spotlight

Harold Ramis and vasculitis

E. Jason Wambsgans, McClatchy-Tribune

E. Jason Wambsgans, McClatchy-Tribune

Leslie Mann, Special to Tribune Newspapers

Thanks to the well-publicized death of actor/director Harold Ramis, the disease he suffered from — vasculitis — is getting more attention, which can help patients and doctors understand this elusive autoimmune disease.

"His death was a tremendous loss, so sad," said Joyce Kullman, executive director of the Kansas City, Mo.-based Vasculitis Foundation. "But it brought a greater awareness to the disease. We've been hearing from people all over the world who are saying, 'Oh my gosh, that's what I have.'"

The foundation estimates about 1 in 3,300 people suffer from vasculitis, which causes the body to attack its own blood cells.

Because vasculitis is a collection of about 15 diseases with seemingly unrelated symptoms, it is difficult to spot. "The typical patient spends months or years getting a diagnosis," said Kullman. "Many patients have organ damage by the time doctors identify it."

Symptoms run the gamut. Patients have chronic sinusitis, joint pain, mouth ulcers, ear infections, itchy eyes, skin lesions or vision problems. If the disease affects the brain, they may have strokelike symptoms such as partial paralysis or headaches. Symptoms start out mild, then get worse or persist.

"Symptoms are often discounted by doctors," said Kullman. "Some people are told it's all in their heads."

"It took me a year to get a diagnosis," said Dianne Shaw, a retired communications director from Chapel Hill, N.C., who has the Wegener's granulomatosis form of the disease. "I was told I had bronchitis, then lung cancer, then valley fever before I was diagnosed correctly in 1995. But I was lucky; I didn't have organ damage yet."

Shaw's vasculitis affects her respiratory system. She has had 36 surgeries or procedures, most of them to clear blocked airways so she can breathe more easily.

The diagnosis delay occurs in part because vasculitis is uncommon, said Dr. Eric Matteson, professor of medicine at Mayo Clinic College of Medicine in Rochester, Minn., by email. "Few physicians are familiar with it," he said.

The Vasculitis Foundation hosts seminars to educate primary-care physicians. "We encourage them to refer patients to specialists," said Kullman.

Ideally, a patient sees a vasculitis team, but there are only 10 listed by the foundation in the United States, at medical centers including Mayo. Each consists of a rheumatologist (autoimmune disease doctor) plus doctors who treat affected organs such as eyes, brain, skin, kidneys, heart or lungs.

"Even patients not near a major medical facility can benefit from an evaluation at a facility specializing in vasculitis, where a plan is developed for management by the patient's home medical team," said Matteson.

Are there triggers for vasculitis? It depends on whom you ask. "Patients say there are — emotional traumas like death of a loved one or physical injuries such as a broken bone — but doctors say they can't prove this scientifically," said Kullman. "We still don't know why an injury or trauma causes one person's immune system to go haywire while another person's is OK."

There is no cure for vasculitis, but treatment is evolving.

"Some (forms of vasculitis) are more treatable than others," said Matteson. "Giant cell arteritis, for example, responds well to treatment, while forms of the disease that affect the brain are extremely difficult to treat."

The biggest news for vasculitis was the 2011 approval by the FDA of a drug called Rituxan, used in combination with steroids. It temporarily relieves blood vessel inflammation, which causes the symptoms.

On the horizon are new procedures and drugs that will better diagnose and treat vasculitis, said Matteson. To get answers, the Vasculitis Foundation funds three new $50,000 studies a year and has 11 underway. "We hope to find a cure," said Kullman. "Or at least identify what causes it."

Matteson encourages patients to read about the disease and network with others in the vasculitis community. "Especially with serious diseases like this one, it is very important for the patient to be as educated as possible about his disease," he said.